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Table 1 Review of the reported ocular problems in Congenital Protein C deficiency

From: Ophthalmic manifestations of congenital protein C deficiency: a case report and mini review

Report Reported cases Involved eye Anterior segment involvement Posterior segment involvement Global involvement
Estelles A et al [1984] [6] 1 OU   Bilateral vitreous hemorrhage and intravitreal mass  
Marciniak E et al. [1985] [7] 1 OU Corneal opacity Case1: Corneal opacity, pupil not visible in one eye
Case2: Bilateral cataract,
Case 1: Vitreous opacity in one eye
Case 2: total retinal detachment with retinal new and old hemorrhages
Microphthalmia
Rappaport ES et al. [1987] [8] 1 OU   Hyperplastic vitreous bilaterally  
Pulido JS [1987] [9] 1 OU Prominent iris vessels, shallow anterior chamber, synechia, cataract, retrolental membrane Bilateral vitreal hemorrhage, and funnel-shaped retinal detachment in both eyes  
Auletta MJ and Headington JT [1988] [10] 1 OU Raised intraocular pressure, flattened anterior chambers, iris atrophy, lens adhesions Bilateral retinal detachment Bilateral leukocoria
Hartman KR [1989] [11] 1 OU   Vitreal eye hemorrhages and Intraparenchymal brain infarction
Hermsen VM et al [1990] [12] 1 OU Vascularized lens, non-existent anterior chambers Funnel-shaped retrolental mass, normal retina and optic nerve Bilateral PHPV, microphthalmos
Soria JM et al. [1985] [13] 1    Persistence of primary vitreous Microphthalmia, irregular globe
Cassels-Brown A et al. [1994] [14] 2 OU Case 1: Posterior embryotoxon, shallow anterior chambers, ectropion uvea, posterior synechia
Case 2: marked periorbital edema and hemorrhagic conjunctival chemosis
Case 1: subconjunctival hemorrhage, a right sided retinal arterial occlusion and bilateral florid retinal hemorrhages with swollen hemorrhagic optic discs secondary to retinal venous occlusions, vitreous hemorrhages retinal detachment in one eye
Case 2: hazy media, minor bilateral vitreous hemorrhages, and minor right sided retinal hemorrhage
Case 1:
Strabismus, nystagmus, leukocoria
Case 2: early birth 25 wk., end up to death at 23 days of life because of some subarachnoid hemorrhage and with worsening lung function
Acheson JF et al. [1994] [15] 4 OD   Bilateral recurrent ischemic optic neuropathy Association with Protein S deficiency, antithrombin III
Dreyfus M et al. [1995] [16] 9 OU    Unilateral or bilateral blindness 6/9 cases bilateral PHPV
Hattenbach LO et al. [1999] [5] 2 OU Case 1. Shallow anterior chamber
Case 2. Shallow anterior chamber, posterior synechia
Case 1. Left retrolental opacities, funnel retinal detachment, vitreous hemorrhage
Case 2. Right vitreous hemorrhage, funnel retinal detachment
Case 1. Strabismus, microphthalmos
Case 2. Microphthalmos
Ergenekon E et al. [2000] [17] 1 OS   Eye ultrasound revealed an10x7 mm hyperechogenic structure underneath.
Retina of the left eye consistent with subretinal hemorrhage
Leukocoria
Churchill AJ et al. [2001] [18] 2 OU Case2. Right leukocoria Case 1. Bilateral central retinal vein occlusions, vitreous hemorrhage, and a right central retinal artery occlusion
Case 2. Total retinal detachment and a left macular hemorrhage
 
Paysse EA [2002] [19] 1 OU Flat anterior chamber Bilateral retinal detachment, fibrotic hyaloid arteries  
Sirachainan N et al [2003] [20] 2 OU    Preserving eye function, high myopia, cerebral palsy
Park UC et al. [2005] [21] 1 OU Bilateral corneal opacity, microphthalmia, posterior synechia, pupillary membrane, shallow anterior chamber Vitreoretinopathy suggesting, PHPV, intravitreal masses, funnel-shaped retinal detachment with bilateral retinal dysplasia Bilateral leukocoria, no microphthalmos, blind OU
de Lemus-Varela ML [2005) [22] 1 OU   Vitreous hemorrhage  
Douglas AG et al. [2010] [23] 1 OU No red reflex OU Vitreoretinal dysplasia, severe hazy media in one eye and OU PFV Leukocoria OS, bilateral microphthalmia,, deranged VEP, poor vision, nystagmus
Wang BZ [2012] [24] 1 OU   Macular hypoplasia with blond retinal background Doll’s eye movements, no fix and follow movement of the eyes, ponto-cerebellar hypoplasia, nystagmus, strabismus, Flat ERG
Desai S et.al. [2014] [25] 1 OU   Combined central retinal venous and arterial obstruction Severe Type II protein C deficiency with factor V Leiden mutation, Glaucoma
Almarzouki HS et al [2016] [26] 2 OU Peter’s anomaly   
Baothman AA et al. [2017] [27] 5 OU Peter’s anomaly, corneal opacity (3/5) Blindness (4/5OU), ocular hemorrhage shortly after birth resulted in visual loss (1/5)  
Present study 1 OU Left eye shallow anterior chamber, cataract, leukoma, posterior synechia Dysplastic retina and total retinal detachment in the other eye Flat ERG, fluorescent spots
  1. ERG Electroretinography, OD Right eye, OS Left eye, OU Both eyes, PHPV Persistent primary hyperplastic vitreous